Companions report seeing more severe Huntington’s signs than patients
Study: New measure captures behavioral symptoms in people with disease
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A new measure, called the Huntington’s Disease-Behavioral Questionnaire (HD-BQ), effectively captures behavioral symptoms in people with Huntington’s disease, as well as differences between patient reports and those of their companions, a study shows.
Companions reported more widespread and severe symptoms than patients, particularly in cognitive domains such as concentration, switching thoughts or activities, judgment, and decisiveness.
“By integrating both patient and companion perspectives, the HD-BQ reveals the impact of anosognosia on behavioral symptom reporting across multiple domains,” researchers wrote. Anosognosia is a neurological condition in which patients fail to recognize their disease and its associated cognitive and physical impairments.
“These findings accentuate that self-report measures alone are insufficient for clinical trials, and that researchers should prioritize the inclusion of collateral informant input,” researchers added.
The study, “Capturing Behavioral Symptoms in Huntington’s Disease Using the Huntington’s Disease-Behavioral Questionnaire,” was published as a brief report in Movement Disorders Clinical Practice by a team of U.S. researchers.
Questionnnaire evaluates 30 common behavioral symptoms
Huntington’s is caused by a mutation in the HTT gene that results in the production of an abnormally long huntingtin protein, which is prone to form toxic clumps in nerve cells. This leads to the dysfunction and death of nerve cells, and consequent movement, cognitive function, and mental health symptoms.
“Despite their clinical relevance, assessments of behavioral symptoms in HD [Huntington’s disease] remain limited in both research and clinical practice,” the researchers wrote, adding that commonly used tools are “time-intensive to administer and largely reflect the clinician’s interpretation rather than the patient’s or caregiver’s perspective.”
To address these limitations, the team previously developed the HD-BQ, a validated measure designed to evaluate 30 common behavioral symptoms across cognitive, functional, and psychiatric categories.
“A distinguishing feature of the HD-BQ is the availability of both self-report and companion-report versions, allowing direct comparison between patient and informant perspectives — a critical capability in populations with impaired insight,” the researchers wrote.
Companions report broader range of behavioral symptoms in patients
In this study, the team evaluated HD-BQ’s clinical utility in Huntington’s by comparing results between patients and healthy controls and by examining differences between patient reports and those of their companions.
A total of 71 adults with Huntington’s and their companions, as well as 71 age-, sex-, and education-matched healthy controls, completed the HD-BQ. Huntington’s participants had a mean age of 54.3 years, and most were women (57.7%).
In HD-BQ, participants rate the presence of 30 behaviors over the past year using a four-point scale from zero (“completely disagree”) to 3 (“completely agree”). Total scores range from zero to 90, with higher scores indicating more severe behavioral issues.
Overall, healthy controls rarely reported behavioral symptoms, resulting in significantly lower HD-BQ scores than those of Huntington’s patients (9 vs. 34). Notably, patients reported significantly greater symptom severity on all but one item.
Companions reported a broader range of behavioral symptoms and significantly higher symptom severity than patients, with a median HD-BQ total score of 43, “reflecting a substantial divergence in the perceived frequency and severity of behavioral symptoms,” the team wrote.
Across all HD-BQ items, the largest difference (26.8%) was observed in the “difficulty shifting thoughts or activities” item, which was frequently referred to by companions but not by patients.
Companions consistently reported more frequent and severe symptoms than patients, likely reflecting patients’ anosognosia.
Other cognitive domains more frequently reported by companions, and with higher severity levels, included difficulty concentrating, poor judgment, difficulty keeping track of commitments, and increased indecisiveness.
Other domains showing significant discrepancies included avoidance of complex activities, excessive daytime sleepiness, agitation, and aggression, which were all more frequently reported or perceived as more severe by companions.
Other symptoms were commonly reported by both Huntington’s patients and their companions. These included taking more time to complete activities (reported by 77.5% of patients and 83.1% of companions), memory problems (64.8% and 76.1%), taking longer to respond (67.6% and 69%), and feeling more anxious (60.6% in both).
Overall, the findings indicated that “companions consistently reported more frequent and severe symptoms than patients, likely reflecting patients’ anosognosia,” the researchers wrote.
While anosognosia has been shown to reduce the accuracy of self-reported psychiatric symptoms in Huntington’s, these “findings extend this effect to behaviors embedded in cognitive and functional domains, consistent with prior work demonstrating that anosognosia spans cognitive, functional, and psychiatric symptoms,” the team concluded.
